Mechanical insufflation/exsufflation improves vital capacity in neuromuscular disorders

Abstract

Inherited neuromuscular disorders inevitably result in severe lung volume restriction associated with high morbidity and mortality. The aim of this retrospective study was to evaluate the long-term effects of the regular use of mechanical insufflation/exsufflation on the course of the vital capacity. This retrospective data analysis included 21 patients (16.1 + 6.5 years) with neuromuscular disorders and severe lung volume restriction using nocturnal noninvasive ventilation. The patients were advised to regularly use the mechanical insufflation/ exsufflation twice a day for 10 minutes applying sets of three insufflation/exsufflation breath via face mask irrespective of respiratory tract infection. Data on the course of vital capacity were collected 2 years prior and 2 years after the introduction of regular use of mechanical insufflation/exsufflation. Before the introduction of mechanical insufflation/exsufflation vital capacity decreased from 0.71 + 0.38 L to 0.50 + 0.24 L in the last year and from 0.88 + 0.45 L to 0.71 + 0.38 L in the next to last year. In the first year, after regular use of mechanical insufflation/exsufflation vital capacity significantly increased by 28% (from 0.50 L to 0.64 L)-after the second year the vital capacity increase remained stable (0.64 vs. 0.65 L). These data suggest that the regular use of mechanical insufflation/exsufflation improves vital capacity in patients with neuromuscular disorders and severe lung volume restriction.

Active lung volume recruitment to preserve vital capacity in Duchenne muscular dystrophy

“…Our observed maximum decline in VC occurring between ages 20 and 21 years can also be compared to the maximum annual rate of decline of 39% and median 8%/year over multiple years reported by Wagner et al for untreated patients (23) and to decreases of 8.5-14%/year over multiple years reported in other studies that identified neither maximum rates of VC decline nor plateau VC (15,16,18,(24)(25)(26)(27)(28)(29). These studies reported decreases in the percentage of predicted VC over ages 10-20 years, of 8.5%/year, or 85% over 10 years (3), 5%/year over ages 5-24 years (n = 60) (26), 8%/year over ages 10-18 years (n = 58) (27,30,31); 10.7%/year over ages 12-15 years (n = 10) (28), and 4.2%/year at mean age 13 years (n = 25) (16).…”

Respiratory Involvement in Patients with Neuromuscular Diseases: A Narrative Review

“…McKim et al also reported retrospectively an 89% decrease in VC decline over a period of 78 months follow-up in 22 DMD patients who were prescribed lung volume recruitment sessions [130]. In another retrospective study on a mixed population of 21 patients with NMDs, the regular use of the MI-E device resulted in improvement in VC by 28% during the first year and stabilization during the second year [131].…”

Respiratory adjuncts to NIV in neuromuscular disease

“…Conversely, a 3‐month, matched‐pair randomized controlled trial (RCT) of inflations in children with DMD found no difference in FVC between those treated with daily IPPB compared to control . Dosage was different between the studies and may explain some of the variability; inflation pressures ranged from 18 to 40 cm H 2 O compared to 10–18 cm H 2 O, and treatment time was 20 versus 6 min per day.…”

“…retrospectively analysed the records of 21 patients with heterogeneous NMD advised to perform b.d. MI‐E incorporating inflations and found an increase in VC in 86% of patients within the first year . Conversely, a 3‐month, matched‐pair randomized controlled trial (RCT) of inflations in children with DMD found no difference in FVC between those treated with daily IPPB compared to control .…”